Osteomas and Dental Abnormalities

Osteomas are the most common accompanying bone lesion seen in FAP, and the first extra-colonic lesion to be associated with FAP [123, 124]. Osteoma is a benign neoplasm of bone tissue that increases in size by slow continuous osseous growth and consists of well-differentiated compact or cancellous bone. It is a painless tumor and has no malignant potential. Since the mandible and skull are the most common locations, asymmetry of the maxillofacial region is typical. In children, it may precede the occurrence of colonic polyposis [125]. Subtle radiopaque jaw lesions are often evident at an early age via panoramic dental radiographs in patients with FAP with no other apparent extra-intestinal lesions [126, 127]. Long bone radiographs may demonstrate osteomas or hyperostosis.

Treatment for bone lesions of FAP depends on the symptomatic or cosmetic nature of the findings. They may require excision if they are severely deforming or interfere with function. In the past, the presence of osteomas was required to make the diagnosis of Gardner syndrome, a variant of FAP. However, with the discovery of the APC gene and the knowledge that FAP and Gardner syndromes arise from mutations of that gene, the term Gardner syndrome remains mainly of historical interest.

Various dental abnormalities have been described in FAP including unerupted teeth, supernumerary teeth, dentigerous cysts and odontomas [128]. These may precede the development of colonic polyposis, and the prevalence is estimated at 17%, compared with 1-2% in the general population.

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